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This page shows you the latest news and research items in this category.

Pleural Mesothelial Cell Differentiation and Invasion in Fibrogenic Lung Injury.
Abstract The origin of the myofibroblast in fibrotic lung disease is uncertain, and no effective medical therapy for fibrosis exists. We have previously demonstrated that transforming growth factor-β1 (TGF-β1) induces pleural mesothelial cell (PMC) transformation into myofibroblasts and haptotactic migration in vitro. Whether PMC differentiation and migration occurs in vivo, and whether this response can be modulated for therapeutic benefit, is unknown. Here, using mice recombinant for green fluorescent protein (GFP) driven by the Wilms tumor-1 (WT-1) promoter, we demonstrate PMC trafficking into the lung and d...
Source: The American Journal of Pathology - February 8, 2013 Category: Pathology Authors: Zolak JS, Jagirdar R, Surolia R, Karki S, Oliva O, Hock T, Guroji P, Ding Q, Liu RM, Bolisetty S, Agarwal A, Thannickal VJ, Antony VB Tags: Am J Pathol Source Type: research

Detection of Preoperative Wilms Tumor Rupture with CT: A Report from the Children's Oncology Group [Pediatric Imaging]
Conclusion: CT has moderate specificity but relatively low sensitivity in the detection of preoperative Wilms tumor rupture. Ascites beyond the cul-de-sac, irrespective of attenuation, is most predictive of rupture. © RSNA, 2012 Supplemental material: http://radiology.rsna.org/lookup/suppl/doi:10.1148/radiol.12120670/-/DC1 (Source: Radiology)
Source: Radiology - January 29, 2013 Category: Radiology Authors: Khanna, G., Naranjo, A., Hoffer, F., Mullen, E., Geller, J., Gratias, E. J., Ehrlich, P. F., Perlman, E. J., Rosen, N., Grundy, P., Dome, J. S. Tags: Pediatric Radiology, Genitourinary Radiology Pediatric Imaging Source Type: research

The Wilms Tumor Gene, Wt1, Maintains Testicular Cord Integrity by Regulating the Expression of Col4a1 and Col4a2.
In this study, we found that the laminin-positive basal lamina lining the testicular cords was fragmented and completely absent in some areas of Wt1(-/flox); Amh-Cre testes, indicating that the testicular cord disruption can be attributed to the breakdown of the basement membrane. To explore the molecular mechanism underlying this effect, we examined the expression of cell adhesion molecules (CAMs) and testicular cord basal lamina components by real-time RT-PCR, western blotting and immunostaining. Compared with control testes, the expression of CAMs (such as E-cadherin, N-cadherin, claudin11, occludin, beta-catenin, and Z...
Source: Biology of Reproduction - January 16, 2013 Category: Reproduction Medicine Authors: Chen SR, Chen M, Wang XN, Zhang J, Wen Q, Ji SY, Zheng QS, Gao F, Liu YX Tags: Biol Reprod Source Type: research

Synchronous bilateral Wilms tumor
CONCLUSIONS:These results provide further evidence that the optimal duration and choice of drugs for preoperative chemotherapy remain an open question. Outcome remained significantly worse for BWT than for unilateral Wilms tumor. To enable the conservative treatment of as many affected kidneys as possible, only centers with experience in BWT should manage such cases. Cancer 2013;. © 2013 American Cancer Society. (Source: Cancer)
Source: Cancer - January 11, 2013 Category: Cancer & Oncology Authors: Paolo Indolfi, Alessandro Jenkner, Monica Terenziani, Alessandro Crocoli, Annalisa Serra, Paola Collini, Davide Biasoni, Lorenza Gandola, Gianni Bisogno, Giovanni Cecchetto, Martina Di Martino, Paolo D'Angelo, Maurizio Bianchi, Massimo Conte, Alessandro I Tags: Original Article Source Type: research

Rare case of blastemal predominant adult Wilms' tumor with skeletal metastasis case report and brief review of literature
We report a case of 19-year-old female presented with severe low backache and colicky left loin pain of 3 months and progressive weakness of 15 days duration. Magnetic resonance image (MRI) of lumbosacral spine was reported as spinal metastasis with right renal mass. The patient underwent right radical nephrectomy and the tumor was histopathologically confirmed as adult Wilms' tumor. In case of adult Wilms' tumor, distant metastasis may be the first presentation and this possibility should be considered when an adult patient presents with flank pain and a renal mass. (Source: Indian Journal of Urology)
Source: Indian Journal of Urology - January 10, 2013 Category: Urology & Nephrology Authors: Rashmi PatnayakD. V. S. RambabuAmitabh JenaBodagala VijaylaxmiBV PhaneendraM Kumaraswamy Reddy Source Type: research

Frasier syndrome: four new cases with unusual presentations
We report here four cases of FS diagnosis after identification of WT1 mutations. Case 1 was part of a large cohort of patients diagnosed with steroid-resistant nephrotic syndrome, in whom the screening for mutations within WT1 8-9 hotspot fragment identified the IVS9+5G>A mutation. Beside FS, this patient showed unusual characteristics, such as urinary malformation (horseshoe kidney), and bilateral dysgerminoma. Cases 2 and 3, also bearing the IVS9+5G>A mutation, and case 4, with IVS9+1G>A mutation, were studied due to FSGS and/or delayed puberty; additionally, patients 2 and 4 developed bilateral gonadal tumors. Since the...
Source: Arquivos Brasileiros de Endocrinologia e Metabologia - January 8, 2013 Category: Endocrinology Source Type: research

The Wilms' tumor gene (WT1) regulates E-cadherin expression and migration of prostate cancer cells
Conclusions: These findings suggested that WT1 expression in high grade prostate cancer may contribute to migration and metastasis. Thus, in prostate cancer WT1 may function as a novel oncogene facilitating development of the lethal metastatic phenotype. (Source: Molecular Cancer)
Source: Molecular Cancer - January 8, 2013 Category: Cancer & Oncology Authors: Adina BrettSony PandeyGail Fraizer Source Type: research

Ubiquitin specific protease 18 (Usp18) is a WT1 transcriptional target.
Abstract Wilms tumor gene WT1 encodes a zinc finger containing transcription factor which is required for renal development. Mutations in WT1 are observed in 20% of Wilms tumor (a pediatric kidney cancer), but the in vivo WT1 targets and associated molecular pathways involved in the etiology of Wilms tumor are still elusive. To identify WT1 targets we performed genome wide comprehensive expression profiling using Affymetrix Gene Chip Mouse Genome 430 2.0 Array, comparing E13.5 mouse kidneys in which Wt1 had been somatically ablated with littermate controls. We identified Usp18 as the most differentially expressed g...
Source: Experimental Cell Research - January 2, 2013 Category: Cytology Authors: Shahidul Makki M, Cristy Ruteshouser E, Huff V Tags: Exp Cell Res Source Type: research

The evolution of treatment for Wilms tumor
Abstract: The prognosis for children with Wilms tumor (WT) has improved dramatically as the result of advances in surgical techniques, anesthesia, and supportive care. During the last three decades, the National Wilms Tumor Study Group (NWTSG), the International Society of Pediatric Oncology (SIOP), and the United Kingdom Children's Cancer Study Group (UKCCSG) conducted sequential studies of treatments for children with WT. The National Wilms Study Group demonstrated that radiation therapy is not necessary for those with Stage I and II, favorable histology Wilms tumor, and that the dose necessary for local control for thos...
Source: Journal of Pediatric Surgery - January 1, 2013 Category: Surgery Authors: Daniel M. Green Tags: Robert E. Gross Lecture Source Type: research

Primary nephrectomy and intraoperative tumor spill: Report from the Children's Oncology Group (COG) renal tumors committee
Abstract: Purpose: Initial Children's Oncology Group (COG) management for Wilms' tumor (WT) consists of primary nephroureterectomy with lymph node sampling. While this provides accurate staging to define further treatment, it may result in intraoperative spill (IOS), which is associated with higher recurrence rates and therefore requires more intensive therapy. The purpose of this study is to determine current rates and identify factors which may predispose a patient to IOS.Methods: The study population was drawn from the AREN03B2 renal tumor banking and classification study of the Children's Oncology Group. All children w...
Source: Journal of Pediatric Surgery - January 1, 2013 Category: Surgery Authors: Kenneth W. Gow, Douglas C. Barnhart, Thomas E. Hamilton, Jessica J. Kandel, Mike K.S. Chen, Fernando A. Ferrer, Mitchell R. Price, Elizabeth A. Mullen, James I. Geller, Eric J. Gratias, Nancy Rosen, Geetika Khanna, Arlene Naranjo, Michael L. Ritchey, Paul Tags: APSA Papers Source Type: research

Historical Trends in the Use of Radiation Therapy for Pediatric Cancers: 1973-2008
Conclusions: The use of RT is declining over time in 7 of 10 pediatric cancer categories. A limitation of this study is a potential under-ascertainment of RT use in the SEER-9 database including the delayed use of RT. (Source: International Journal of Radiation Oncology * Biology * Physics)
Source: International Journal of Radiation Oncology * Biology * Physics - December 28, 2012 Category: Radiology Authors: Vikram Jairam, Kenneth B. Roberts, James B. Yu Tags: Pediatric Cancers Source Type: research

Verrucous Hemangioma Expresses Primitive Markers
This study characterized the cell population within verrucous hemangioma. Material and MethodsParaffin‐embedded sections were verrucous hemangioma from two male patients were processed for immunohistochemistry. The expression of SMA, CD34, Glut‐1, D2‐40, brachyury, angiotensin converting enzyme (ACE), Oct‐4, hemoglobin ζ chain (HBZ), Wilms tumor protein (WT‐1) and CD45 were examined. ResultsThe lymphatic marker, D2‐40, was not expressed by verrucous hemangioma. While Glut‐1 was widely expressed in infantile hemangioma, it was only focally expressed by the endothelium of verrucous hemangioma. The endothelium ...
Source: Journal of Cutaneous Pathology - December 27, 2012 Category: Pathology Authors: Emma L Laing, Helen D Brasch, Ryan Steel, Jun Jia, Tinte Itinteang, Swee T Tan, Darren J Day Tags: Original Article Source Type: research

Survivin selective inhibitor YM155 induce apoptosis in SK-NEP-1 Wilms tumor cells
Conclusions: The present study demonstrates that YM155 treatment resulted in apoptosis and inhibition of cell proliferation of SK-NEP-1cells. YM155 had significant role and little side effect in the treatment of SK-NEP-1 xenograft tumors. Real-time PCR array analysis firstly showed expression profile of genes dyes-regulated after YM155 treatment. IPA analysis also represents new molecule mechanism of YM155 treatment, such as NR3C1 and dexamethasone may be new target of YM155. And our results may provide new clues of molecular mechanism of apoptosis induced by YM155. (Source: BMC Cancer)
Source: BMC Cancer - December 26, 2012 Category: Cancer & Oncology Authors: Yan-Fang TaoJun LuXiao-Juan DuLi-Chao SunXuan ZhaoLiang PengLan CaoPei-Fang XiaoLi PangDong WuNa WangXing FengYan-Hong LiJian NiJian WangJian Pan Source Type: research

Editorial Comment
Although Wilms tumor stage, histology and other clinical factors are important proxies for tumor aggressiveness, unfortunately they are not perfect. Thus, clinicians must use imperfect methods to balance the risk of tumor recurrence against the risk of treatment related morbidity. Biomarkers are assuming an increasingly important place in Wilms tumor treatment regimens as investigators seek to improve our ability to risk stratify our young patients. (Source: The Journal of Urology)
Source: The Journal of Urology - December 20, 2012 Category: Urology & Nephrology Authors: Jonathan C. Routh Tags: Pediatric Urology Source Type: research

Children's Oncology Group's 2013 blueprint for research: Renal tumors
Abstract Renal malignancies are among the most prevalent pediatric cancers. The most common is favorable histology Wilms tumor (FHWT), which has 5‐year overall survival exceeding 90%. Other pediatric renal malignancies, including anaplastic Wilms tumor, clear cell sarcoma, malignant rhabdoid tumor, and renal cell carcinoma, have less favorable outcomes. Recent clinical trials have identified gain of chromosome 1q as a prognostic marker for FHWT. Upcoming studies will evaluate therapy adjustments based on this and other novel biomarkers. For high‐risk renal tumors, new treatment regimens will incorporate biological ther...
Source: Pediatric Blood and Cancer - December 19, 2012 Category: Cancer & Oncology Authors: Jeffrey S. Dome, Conrad V. Fernandez, Elizabeth A. Mullen, John A. Kalapurakal, James I. Geller, Vicki Huff, Eric J. Gratias, David B. Dix, Peter F. Ehrlich, Geetika Khanna, Marcio H. Malogolowkin, James R. Anderson, Arlene Naranjo, Elizabeth J. Perlman, Tags: Review Source Type: research

A Wt1-Dmrt1 Transgene Restores DMRT1 to Sertoli Cells of Dmrt1-/- Testes: A Novel Model of DMRT1-Deficient Germ Cells.
This study characterized a novel mouse model of DMRT1-deficient germ cells that was generated by breeding Dmrt1-null (Dmrt1(-/-)) mice with Wt1-Dmrt1 transgenic (Dmrt1(+/-;tg)) mice, which express a rat Dmrt1 cDNA in gonadal supporting cells by directing it from the Wilms tumor locus in a yeast artificial chromosome transgene. Like Dmrt1(-/-) mice, male Dmrt1(-/-) transgenic mice (Dmrt1(-/-;tg)) were infertile, while female mice were fertile. Immunohistochemistry and Western blot analysis showed transgenic DMRT1 expressed in supporting cells of the newborn gonads of both sex and in Sertoli cells of the testis afterbirth. S...
Source: Biology of Reproduction - December 19, 2012 Category: Reproduction Medicine Authors: Agbor VA, Tao S, Lei N, Heckert LL Tags: Biol Reprod Source Type: research

Constitutional and somatic methylation status of DMRH19 and KvDMR in Wilms tumor patients
The most frequent epigenetic alterations in Wilms tumor (WT) occur at WT2, assigned to 11p15. WT2 consists of two domains: telomeric domain 1 (DMRH19) that contains the IGF2 gene and an imprinted maternally expressed transcript (H19) and centromeric domain 2 (KvDMR) that contains the genes KCNQ1, KCNQ1OT1 and CDKN1C. In this work, we used pyrosequencing and MS-MLPA to compare the methylation patterns of DMRH19/KvDMR in blood and tumor samples from 40 WT patients. Normal constitutional KvDMR methylation indicated that most of the epigenetic alterations in WT occur at DMRH19. Constitutional DMRH19 hypermethylation (HM DMRH19...
Source: Genetics and Molecular Biology - December 15, 2012 Category: Genetics & Stem Cells Source Type: research

Relapse of unilateral favorable histology Wilms’ tumor: Significant clinicopathological factors
Conclusion: According to the basic Japanese therapeutic strategy, all patients underwent a primary nephrectomy before chemotherapy. This study suggests that the histological subtype pre-treatment “BPT-WT” should be included as a strong indicator of poor prognosis. Such patients should be treated as a high-risk group. (Source: Journal of Pediatric Surgery)
Source: Journal of Pediatric Surgery - December 1, 2012 Category: Surgery Authors: Takeshi Aoba, Naoto Urushihara, Koji Fukumoto, Shigeyuki Furuta, Hiroaki Fukuzawa, Maki Mitsunaga, Kentaro Watanabe, Masaya Yamoto, Hiromu Miyake, Mariko Koyama, Hideto Iwabuchi, Junki Koike, Shinobu Tatsunami, Munechika Wakisaka, Hiroaki Kitagawa Tags: PAPS Papers Source Type: research

CITED1 Expression in Liver Development and Hepatoblastoma.
Abstract Hepatoblastoma, the most common pediatric liver cancer, consists of epithelial mixed embryonal/fetal (EMEF) and pure fetal histologic subtypes, with the latter exhibiting a more favorable prognosis. Few embryonal histology markers that yield insight into the biologic basis for this prognostic discrepancy exist. CBP/P-300 interacting transactivator 1 (CITED1), a transcriptional co-activator, is expressed in the self-renewing nephron progenitor population of the developing kidney and broadly in its malignant analog, Wilms tumor (WT). In this current study, CITED1 expression is detected in mouse embryonic liv...
Source: Neoplasia - December 1, 2012 Category: Cancer & Oncology Authors: Murphy AJ, de Caestecker C, Pierce J, Boyle SC, Ayers GD, Zhao Z, Libes JM, Correa H, Walter T, Huppert SS, Perantoni AO, de Caestecker MP, Lovvorn HN Tags: Neoplasia Source Type: research

The significance of Wilms Tumor Gene (WT1) and p53 expression in curettage specimens of patients with endometrial carcinomas.
Abstract In this retrospective experimental study, we assessed the immunohistochemical expression of Wilms Tumor Gene (WT1) and p53 in endometrial biopsies of patients with endometrial cancer, and correlated their expression with the final pathological findings. Sixty-two patients with primary endometrial cancer who underwent surgical treatment were investigated. Immunohistochemical expression of Wilms Tumor Gene (WT1) and p53 was assessed in curettage specimens, and the final pathology reports from hysterectomy specimens were reviewed. The expression of these markers seems to play a role in curettage specimens as ...
Source: Pathology, Research and Practice - November 30, 2012 Category: Pathology Authors: Matalka I, Obeidat B, Mohtaseb A, Awamleh A Tags: Pathol Res Pract Source Type: research

Detection of Preoperative Wilms Tumor Rupture with CT: A Report from the Children's Oncology Group [Pediatric Imaging]
The presence of ascites beyond the cul-de-sac, irrespective of attenuation, is the most useful CT indicator of preoperative Wilms tumor rupture; in the presence of ascites, fat stranding around the tumor and the presence of retroperitoneal fluid are also highly predictive of rupture. (Source: Continuous Publishing articles)
Source: Continuous Publishing articles - November 28, 2012 Category: Radiology Authors: Khanna, G., Naranjo, A., Hoffer, F., Mullen, E., Geller, J., Gratias, E. J., Ehrlich, P. F., Perlman, E. J., Rosen, N., Grundy, P., Dome, J. S. Tags: Pediatric Radiology, Genitourinary Radiology Pediatric Imaging Source Type: research

Reply by Authors
Dealing with identification of new prognostic biomarkers in a disease as rare as Wilms tumor, for which the overall survival rate is high—and even higher for some subgroups of children—is challenging. Using current clinical and histological classification systems, the delicate process of decreasing therapy related morbidity while preventing relapses has probably reached a plateau. The limitations on drawing any conclusions from our analysis relate not only to the small sample size, but also to the relatively small number of events in terms of tumor recurrences and deaths. This situation is unavoidable for a rare diseas...
Source: The Journal of Urology - November 20, 2012 Category: Urology & Nephrology Tags: Pediatric Urology Source Type: research

Editorial Comment
Cancer is a horrible disease and Wilms tumor is no exception. Undoubtedly the therapeutic advances of recent years have resulted in satisfactory survival rates. Nevertheless, the condition and its management are still associated with important morbidity and adverse outcomes. Clearly we must continue to work toward even better treatment strategies. (Source: The Journal of Urology)
Source: The Journal of Urology - November 20, 2012 Category: Urology & Nephrology Authors: Rodrigo Romao, Armando J. Lorenzo Tags: Pediatric Urology Source Type: research

Loss of Heterozygosity Analysis at Different Chromosome Regions in Wilms Tumor Confirms 1p Allelic Loss as a Marker of Worse Prognosis: A Study from the Italian Association of Pediatric Hematology and Oncology
Conclusions: Chromosome 1p loss of heterozygosity seems to be a risk factor for nonanaplastic Wilms tumor, possibly regardless of other clinical factors. Our findings were uninformative regarding loss of heterozygosity in the other chromosomal regions tested. (Source: The Journal of Urology)
Source: The Journal of Urology - November 20, 2012 Category: Urology & Nephrology Authors: Filippo Spreafico, Beatrice Gamba, Luigi Mariani, Paola Collini, Paolo D'Angelo, Andrea Pession, Andrea Di Cataldo, Paolo Indolfi, Marilina Nantron, Monica Terenziani, Carlo Morosi, Paolo Radice, Daniela Perotti, AIEOP Wilms Tumor Working Group Tags: Pediatric Urology Source Type: research

Ectopic immature renal tissue: clues for diagnosis and management.
We report a 1-year-old male patient who underwent surgery for a sacral subcutaneous small teratoma with a prevalent component of immature renal tissue. The lesion appeared completely excised and, in absence of features of malignancy, only follow-up was suggested. The patient was alive and well 15 months postoperatively. Whenever ectopic immature renal tissue is detected, a proper histological interpretation is mandatory, in order to plan a suitable treatment of the patient. From an extensive analysis of cases reported in literature we draw some practical suggestions for the diagnosis and treatment of this rare condition. ...
Source: International Journal of Clinical and Experimental Pathology - November 15, 2012 Category: Pathology Authors: Coli A, Angrisani B, Chiarello G, Massimi L, Novello M, Lauriola L Tags: Int J Clin Exp Pathol Source Type: research

Adolescent Urologic oncology: Current issues and future directions.
This article will serve to review the most pertinent adolescent urologic oncologic diagnoses (testicular germ call malignancy, the second peak of the bimodal age distribution of GU-RMS, and adolescent renal malignancies). Also, we focus on such issues as the therapeutic impact on fertility, radiation exposure during therapy, and surveillance, risk of secondary malignancy, the long-term impact of chemotherapy, and the psychosocial burden of cancer in this population. Lastly, we highlight future directions and the foreseeable obstacles towards achieving the same research and therapeutic success enjoyed in pediatric urologic ...
Source: Urologic Oncology - November 7, 2012 Category: Urology & Nephrology Authors: Cost NG, Cost CR, Geller JI, Defoor WR Tags: Urol Oncol Source Type: research

The Igf2as Transcript is Exported into Cytoplasm and Associated with Polysomes.
The Igf2as Transcript is Exported into Cytoplasm and Associated with Polysomes. Biochem Genet. 2012 Oct 30; Authors: Duart-Garcia C, Braunschweig MH Abstract Murine insulin-like growth factor 2 antisense (Igf2as) transcripts originate from the opposite strand of the same Igf2 locus as the Igf2 sense mRNA. The Igf2, insulin 2 (Ins2), and H19 genes form a cluster of imprinted genes on chromosome 7. Loss of imprinting of IGF2 in humans is associated with Beckwith-Wiedemann syndrome and Silver-Russell syndrome, as well as with Wilm's tumor and colorectal cancer. We developed a RNA-FISH protocol to detect Igf2as...
Source: Biochemical Genetics - October 30, 2012 Category: Genetics & Stem Cells Authors: Duart-Garcia C, Braunschweig MH Tags: Biochem Genet Source Type: research

Effect of the best compatibility of components in Corni Fructus on WT1 expression in glomerular podocytes of type 2 diabetic rats with early nephropathy.
In this study, the effect of PC on WT1 expression in glomerular podocytes and the mechanism of PC on early nephropathy in type 2 diabetic rats have been investigated. Type 2 diabetic rats were generated by high-fat diet combined with intraperitoneal injection of 30 mg/kg streptozotocin (STZ), then fed aminoguanidine (100 mg/kg), glimepiride (0.4 mg/kg), low-dose (60 mg/kg) or high-dose (120 mg/kg) of PC once a day for 12 weeks. Fasting blood glucose (FBG) was examined regularly. Insulin (INS) was measured by a radioimmunoassay. Microalbuminuria (mALB) was measured by an ELISA assay. Urinary creatinine (UCr), blood urea nit...
Source: The American Journal of Chinese Medicine - October 29, 2012 Category: Complementary Medicine Authors: Liu H, Xu H, Shen C, Wu C Tags: Am J Chin Med Source Type: research

Rhabdomyomatous differentiation in wilms tumor pulmonary metastases: a case report and literature review.
We report an unusual case of Wilms tumor in a 21- year-old man with rhabdomyomatous differentiation of pulmonary metastases after chemotherapy, which presented a challenge during frozen section diagnosis. PMID: 23090738 [PubMed - in process] (Source: Annals of Clinical and Laboratory Science)
Source: Annals of Clinical and Laboratory Science - October 27, 2012 Category: Laboratory Medicine Authors: Seifert RP, McNab P, Sexton WJ, Sawczyn KK, Smith P, Coppola D, Bui MM Tags: Ann Clin Lab Sci Source Type: research

Get on the map!
Join KidneyCancer.ME to meet other families near you that are dealing with renal cancers. Sign up with Facebook. Once you're a part of the newest virtual community for those whose lives have been touched by kidney cancer (including Wilms tumor that affects children), you'll be able to see yourself and others on a map. Then, you may connect with others in your neighborhood or across the nation that are dealing with renal cancers. In the Forum section you can initiate topical discussions on any aspect of kidney cancer that interests you. Get on the map today at www.KidneyCancer.Me. 10/22/2012 (Source: Kidney Cancer Association)
Source: Kidney Cancer Association - October 22, 2012 Category: Cancer & Oncology Source Type: news

Expression and roles of Wilms' tumor 1‐associating protein in glioblastoma
Glioblastoma is a diffusely growing malignant brain tumor and among the most aggressive of all tumors. Wilms' tumor 1‐associating protein (WTAP) is a nuclear protein that has been associated with regulation of proliferation and apoptosis. Although its dynamic expression and physiological functions in vascular cells have been reported, those in other cells are largely unknown. Here, we show for the first time that WTAP is overexpressed in glioblastoma. Moreover we found that WTAP regulates migration and invasion of glioblatoma cells. Specific knockdown by siRNA or overexpression by cDNA regulated migration and invasion of...
Source: Cancer Science - October 22, 2012 Category: Cancer & Oncology Authors: Du‐Il Jin, Sang Weon Lee, Myoung‐Eun Han, Hyun‐Jung Kim, Seon‐Ae Seo, Gi‐Yeong Hur, Shin Jung, Bong‐Seon Kim, Sae‐Ock Oh Tags: Original Article Source Type: research

A Novel Mouse Model of Podocyte Depletion.
Conclusion: The glomerulus has a significant capacity for repair after a podocyte-depleting injury. PMID: 23095233 [PubMed - as supplied by publisher] (Source: Nephron Experimental Nephrology)
Source: Nephron Experimental Nephrology - October 19, 2012 Category: Urology & Nephrology Authors: Wang L, Tang Y, Howell DN, Ruiz P, Spurney RF Tags: Nephron Exp Nephrol Source Type: research

Global demethylation in loss of imprinting subtype of wilms tumor
Abstract Epigenetic abnormalities at the IGF2/H19 locus play a key role in the onset of Wilms tumor. These tumors can be classified into three molecular subtypes depending on the events occurring at this locus: loss of imprinting (LOI), loss of heterozygosity (LOH), or retention of imprinting (ROI). As IGF2 LOI is a consequence of aberrant methylation, we hypothesized that this subtype of Wilms tumors might display global abnormalities of methylation. We therefore analyzed the methylation status of satellite DNA, as a surrogate for global methylation in 50 Wilms tumor patients. Satellite methylation was quantified by a met...
Source: Genes, Chromosomes and Cancer - October 17, 2012 Category: Cancer & Oncology Authors: Jackie L. Ludgate, Gwenn Le Mée, Ryuji Fukuzawa, Euan J. Rodger, Robert J. Weeks, Anthony E. Reeve, Ian M. Morison Tags: Research Article Source Type: research

Quantitative monitoring of WT1 expression in peripheral blood before and after allogeneic stem cell transplantation for acute myeloid leukemia - a useful tool for early detection of minimal residual disease.
Abstract Overexpressed Wilms tumor gene 1 (WT1) has been found in a majority of patients with acute myeloid leukemia (AML). The aim of this study was to confirm the applicability of WT1 expression measurement as a marker of minimal residual disease (MRD). The expression of WT1 gene was measured by real-time polymerase chain reaction in peripheral blood (PB) according to European Leukemia Net (ELN) recommendations. The WT1 expression was related to the expression of a reference gene Abelson (ABL) and the results were calculated as a number of WT1 copies related to 104 copies of ABL gene. The upper normal lim...
Source: Neoplasma - October 16, 2012 Category: Cancer & Oncology Authors: Polak J, Hajkova H, Haskovec C, Cechova H, Marinov I, Mikulenkova D, Markova J, Markova M, Vitek A, Valkova V Tags: Neoplasma Source Type: research

Primary mucinous cystadenoma of the spermatic cord within the inguinal canal
We report a hitherto not documented case of primary mucinous cystadenoma arising in thespermatic cord within the right inguinal canal of a78-year-old man. The tumor was painless,hard and mobile. A computed tomography scan on the pelvis revealed an oval shaped, lowattenuation mass, measuring 5.0x2.5x2.1 cm, that was present adjacent to the vas deferens.Grossly, the excised mass was multicystic mucinous tumor, filled with thick mucoidmaterials. Microscopically, the cystic wall was irregularly thickened. The cystic epitheliumcommonly showed short papillae lined by a single layer of columnar to cuboidal mucinousepithelial cell...
Source: Diagnostic Pathology - October 8, 2012 Category: Pathology Authors: Jee-Yeon KimYoung-Taek LeeHyun-Jeong KangChang-Hun Lee Source Type: research

p53 Immunohistochemistry Expression in Wilms Tumor: A Prognostic Tool in the Detection of Tumor Aggressiveness
Purpose: We studied whether immunohistochemical expression of p53 in Wilms tumors correlates with tumor aggressiveness. We also examined whether preoperative chemotherapy results in any alteration of p53 expression. Materials and Methods: A total of 18 patients underwent preoperative chemotherapy and 30 underwent immediate surgery for Wilms tumor. All children were younger than 10 years and had histologically confirmed disease. Patients with a bilateral tumor or a syndrome related to Wilms tumor were excluded. All pathology slides were uniformly stained for p53 protein, and p53 staining density and intensity were scored...
Source: The Journal of Urology - October 2, 2012 Category: Urology & Nephrology Authors: Jan Franken, Evelyne Lerut, Hendrik Van Poppel, Guy Bogaert Tags: Pediatric Urology Source Type: research

Pelvic Wilms tumor in a child with an absent right kidney and spinal malformations
We report a very rare association of multiple spinal malformations, right kidney absence, and pelvic Wilms tumor in a 21-month-old girl. The report includes a brief review of relevant literature. (Source: Journal of Pediatric Surgery)
Source: Journal of Pediatric Surgery - October 1, 2012 Category: Surgery Authors: Kai Li, Xianmin Xiao, Jiechun Gao, Wei Yao, Hong Chen, Bin Zhang Tags: Journal of Pediatric Surgery Electronic Pages (Available only online at www.jpedsurg.org) Source Type: research

Effects of cyclic increase in gonadotropins on the in vitro development of primordial follicles to antral stage.
Abstract The aim of this study was to evaluate the effects of FSH and LH on follicle development during a long-term culture of cryopreserved human ovarian tissue, using morphological and ultrastructural examinations. Thawed ovarian tissue slices from a 4-year-old child with Wilms tumor were cultured for 32 weeks in two different culture conditions, without (medium A) and with (medium B) a monthly peaked increase in FSH and LH. At week 32, in the medium B cultured tissue, a cluster of preantral follicles associated with two oocytes prematurely ovulated was observed, suggesting that the cyclic increase of gonadotropi...
Source: Ultrastructural Pathology - October 1, 2012 Category: Pathology Authors: Fabbri R, Pasquinelli G, Parazza I, Macciocca M, Magnani V, Battaglia C, Paradisi R, Venturoli S Tags: Ultrastruct Pathol Source Type: research

Treatment of Pulmonary Metastases in Children With Stage IV Nephroblastoma With Risk-Based Use of Pulmonary Radiotherapy [Pediatric Oncology]
Conclusion Following the SIOP protocol, pulmonary RT can be omitted for a majority of patients with PM and results in a relatively good outcome. (Source: Journal of Clinical Oncology)
Source: Journal of Clinical Oncology - September 28, 2012 Category: Cancer & Oncology Authors: Verschuur, Tinteren, Graf, Bergeron, Sandstedt, de Kraker Tags: Renal Tumors, Wilms' Tumor: Therapy, Radiation Oncology Pediatric Oncology Source Type: research

Re: Wilms' Tumor Metastatic to Bilateral Testes at Presentation: Case and Review of the Literature
B. W. Palmer, Y. Xiong, A. Gherezghiher, D. Buethe, A. Metwalli, D. Frimberger and B. P. Kropp Department of Pediatric Urology, University of Oklahoma, Oklahoma City, Oklahoma (Source: The Journal of Urology)
Source: The Journal of Urology - September 21, 2012 Category: Urology & Nephrology Authors: Douglas A. Canning Tags: Pediatric Urology Source Type: research

Bilateral, multicenteric metanephric adenoma associated with Wilms' tumor in a child: A rare presentation with important diagnostic and therapeutic implications
We describe a rare case of bilateral, multicentric metanephric adenoma associated with triphasic Wilms' tumor (stage II) of the left kidney in a male child. (Source: International Journal of Urology)
Source: International Journal of Urology - August 28, 2012 Category: Urology & Nephrology Authors: Sunil Pasricha, Jatin S Gandhi, Gurudutt Gupta, Anurag Mehta, Shaham Beg Source Type: research

Fatal Ischemic Enteritis with Hemorrhage-A Late Complication of Treated Wilms Tumor.
Abstract A 35-year-old man with a history of childhood Wilms tumor successfully treated with radiotherapy, chemotherapy, and surgery, collapsed and died unexpectedly in hospital following admission for abdominal pain. At autopsy, there was ischemic necrosis of the small intestine with altered blood within the stomach and small intestine. Within the upper abdominal aorta, there was patchy confluent calcific atherosclerosis with extension into the proximal superior mesenteric artery which was occluded by thrombus. Death was attributed to ischemic enteritis of the small intestine caused by mesenteric artery thrombosis...
Source: Journal of Forensic Sciences - August 24, 2012 Category: Forensic Medicine Authors: Gilbert JD, Byard RW Tags: J Forensic Sci Source Type: research

Mapping of novel peptides of WT-1 and presenting HLA alleles that induce epitope-specific HLA-restricted T cells with cytotoxic activity against WT-1+ leukemias
We report 41 previously unreported epitopes of WT-1: 5 presented by class II and 36 by class I alleles, including 10 that could be presented by more than 1 class I allele. IFN+ T cells responding to 98% of the class I and 60% of the class II epitopes exhibited HLA-restricted cytotoxicity against peptide-loaded targets. T cells specific for 36 WT-1 peptides were evaluable for leukemocidal activity, of which 27 (75%) lysed WT-1+ leukemic targets sharing their restricting HLA allele. Each epitope identified induced T-cell responses in most donors sharing the epitopes' presenting allele; these responses often exceeded response...
Source: Blood - August 23, 2012 Category: Hematology Authors: Doubrovina, E., Carpenter, T., Pankov, D., Selvakumar, A., Hasan, A., O'Reilly, R. J. Tags: Immunobiology Source Type: research

Favorable response of heavily treated wilms' tumor to Paclitaxel and Carboplatin.
Conclusion: In a refractory case after intensive treatments, we succeeded to control the disease for a while. PMID: 22868510 [PubMed - in process] (Source: Onkologie)
Source: Onkologie - August 20, 2012 Category: Cancer & Oncology Authors: Ozaki S, Takigawa N, Ichihara E, Hotta K, Oze I, Kurimoto E, Fushimi S, Ogino T, Tabata M, Tanimoto M, Kiura K Tags: Onkologie Source Type: research

Analysis of mutational status, SNP rs16754, and expression levels of Wilms tumor 1 (WT1) gene in acute promyelocytic leukemia
Abstract Overexpression, polymorphisms, and mutations of the WT1 gene have been reported in several human tumors including acute myeloid leukemia (AML) and variably correlated with prognosis. Acute promyelocytic leukemia (APL) represents the AML subset disclosing higher WT1 expression levels; however, no WT1 studies specifically focused on APL have been conducted. We screened for the presence of mutations, SNP rs16754, and expression levels of WT1 gene in 103 adult patients with newly diagnosed APL. Fms-like tyrosine kinase (FLT3) mutations were analyzed as well. WT1 mutations were identified in four (4 %...
Source: Annals of Hematology - August 16, 2012 Category: Hematology Tags: Annals of Hematology Source Type: research

Juxtarenal Wilms Tumor in an Adolescent
We present the first case of a juxtarenal Wilms tumor described in an adolescent. (Source: Urology)
Source: Urology - August 16, 2012 Category: Urology & Nephrology Authors: Jennifer Gordetsky, Philip Katzman, Hani Rashid Tags: Pediatric Case Reports Source Type: research

Extrarenal Wilms' Tumor of the Ovary: A Case Report and Short Review of the Literature
Journal of Gynecologic Surgery Aug 2012, Vol. 28, No. 4: 306-308. (Source: Journal of Gynecologic Surgery)
Source: Journal of Gynecologic Surgery - August 14, 2012 Category: OBGYN Tags: article Source Type: research

Metastatic medulloblastoma in an adolescent with Simpson–Golabi–Behmel syndrome
We describe the case of a 12‐year‐old Hispanic male with a clinical and molecular diagnosis of Simpson–Golabi–Behmel Syndrome (SGBS) who subsequently developed metastatic medulloblastoma. While individuals with SGBS have been documented to have increased risk for intra‐abdominal tumors such as Wilms tumor and neuroblastoma, medulloblastomas, or CNS tumors in general, have not been reported in patients with this syndrome. Our patient was clinically diagnosed with SGBS as an infant. He presented with many of the common features of the syndrome, such as cleft palate, macroglossia, post‐axial polydactyly, “coarse...
Source: American Journal of Medical Genetics Part A - August 14, 2012 Category: Genetics & Stem Cells Authors: Martha Thomas, Victoria Enciso, Robert Stratton, Shafqat Shah, Thomas Winder, Marwan Tayeh, Elizabeth Roeder Tags: Clinical Report Source Type: research

HIF-1α, VEGF and WT-1 are protagonists in bilateral primary angiosarcoma of breast: a case report and review of literature.
In conclusion, HIF- 1α, WT-1 and VEGF are possible protagonists in the development of bilateral primary angiosarcoma of breast. The neoplastic process involves endothelial cell of blood vessels lineage rather than lymphatic lineage. Painless breast tumors in young women that are highly vascular at the time of biopsy should be considered as malignant until proven otherwise. Tissue biopsy is the gold standard in the diagnosis of primary angiosarcoma of breast. PMID: 22558480 [PubMed - in process] (Source: International Journal of Clinical and Experimental Pathology)
Source: International Journal of Clinical and Experimental Pathology - August 10, 2012 Category: Pathology Authors: Al-Salam S, Balalaa N, Faour I, Akhter S, Alashari M Tags: Int J Clin Exp Pathol Source Type: research

Monitoring Therapy with MEK Inhibitor U0126 in a Novel Wilms Tumor Model in Wt1 Knockout Igf2 Transgenic Mice Using 18F-FDG PET with Dual-Contrast Enhanced CT and MRI: Early Metabolic Response Without Inhibition of Tumor Growth
Conclusions The optimized dual contrast PET/CT imaging with early post i.v. and i.p. contrast CT and 3 h delayed PET imaging after 18F-FDG administration provides a sensitive and reliable method for detecting early tumor lesions in this endogenous mouse model of Wilms tumor and for monitoring their growth in response to targeted therapies. Therapy with MEK inhibitor U0126 produces only a transient inhibition of tumor glycolytic activity but does not inhibit tumor growth, which is due to continuing IGF2-induced signaling from IGF1R through the PI3K-AKT-mTOR pathway. Content Type Journal ArticleCa...
Source: Molecular Imaging and Biology - August 9, 2012 Category: Molecular Biology Tags: Molecular Imaging and Biology Source Type: research